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Overexpression of innate immune response genes in a model of recessive polycystic kidney disease.

Authors: Mrug, M  Zhou, J  Woo, Y  Cui, X  Szalai, AJ  Novak, J  Churchill, GA  Guay-Woodford, LM 
Citation: Mrug M, etal., Kidney Int. 2008 Jan;73(1):63-76. Epub 2007 Oct 24.
Pubmed: (View Article at PubMed) PMID:17960140
DOI: Full-text: DOI:10.1038/sj.ki.5002627

Defects in the primary cilium/basal body complex of renal tubular cells cause polycystic kidney disease (PKD). To uncover pathways associated with disease progression, we determined the kidney transcriptome of 10-day-old severely and mildly affected cpk mice, a model of recessive PKD. In the severe phenotype, the most highly expressed genes were those associated with the innate immune response including many macrophage markers, particularly those associated with a profibrotic alternative activation pathway. Additionally, gene expression of macrophage activators was dominated by the complement system factors including the central complement component 3. Additional studies confirmed increased complement component 3 protein levels in both cystic and non-cystic epithelia in the kidneys of cpk compared to wild-type mice. We also found elevated complement component 3 activation in two other mouse-recessive models and human-recessive PKD. Our results suggest that abnormal complement component 3 activation is a key element of progression in PKD.

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CRRD Object Information
CRRD ID: 7175542
Created: 2012-12-07
Species: All species
Last Modified: 2012-12-07
Status: ACTIVE



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RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.